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Gastrointestinal symptoms are common in patients with cancer, whether related to treatment or a direct effect of the disease itself. Patients may choose to access cannabinoids outside of their formal medical prescriptions to palliate such symptoms. However, clinical guidelines are lacking in relation to the use of such medicines for gastrointestinal symptoms in patients with cancer.A systematic review of the evidence for the use of cannabinoids for symptom control in patients with cancer was undertaken. Search strategies were developed for Medline, Embase, PsychINFO, and the Cochrane Central Register of Controlled Trials, including all publications from 1975 up to 12 November 2021. Studies were included if they were randomized controlled trials of cannabinoids compared with placebo or active comparator in adult patients with cancer, regardless of type, stage, or treatment status. Articles for inclusion were agreed by all authors, and data extracted and summarized by two authors. Each study was scored according to the Jadad scale. This review was specifically for the purpose of developing guidelines for the use of cannabis for gastrointestinal symptoms, including chemotherapy-induced nausea and vomiting (CINV), chronic nausea, anorexia-cachexia syndrome, and taste disturbance.Thirty-six randomized controlled trials were identified that met the inclusion criteria for this review of gastrointestinal symptoms: 31 relating to CINV, one to radiotherapy-induced nausea and vomiting, and the remaining four to anorexia-cachexia and altered chemosensory disturbance. The populations for the randomized controlled trials were heterogeneous, and many studies were of poor quality, lacking clarity regarding method of randomization, blinding, and allocation concealment. For CINV, eleven RCTs showed improvement with cannabis compared to placebo, but out of 21 trials where cannabis was compared to other antiemetics for CINV, only 11 favoured cannabis.Tetrahydrocannabinol (THC) and nabilone were more effective in preventing CINV when compared to placebo but are not more effective than other antiemetics. For refractory CINV, one study of THC:CBD demonstrated reduced nausea as an add-on treatment to guideline-consistent antiemetic therapy without olanzapine. The MASCC Guideline Committee found insufficient evidence to recommend cannabinoids for the management of CINV, nausea from advanced cancer, cancer-associated anorexia-cachexia, and taste disturbance. High-quality studies are needed to inform practice.

Palliative care has evolved to be an integral part of comprehensive cancer care with the goal of early intervention to improve quality of life and patient outcomes. The NCCN Guidelines for Palliative Care provide recommendations to help the primary oncology team promote the best quality of life possible throughout the illness trajectory for each patient with cancer. The NCCN Palliative Care Panel meets annually to evaluate and update recommendations based on panel members’ clinical expertise and emerging scientific data. These NCCN Guidelines Insights summarize the panel’s recent discussions and highlights updates on the importance of fostering adaptive coping strategies for patients and families, and on the role of pharmacologic and nonpharmacologic interventions to optimize symptom management.

Objectives Our study sought to further characterize patterns of medical cannabis use in elderly cancer patients. Furthermore, we sought to assess efficacy of medical cannabis for the treatment of pain, nausea, anorexia, insomnia and anxiety in elderly cancer patients. Background Medical cannabis use is growing for symptom management in cancer patients, but limited data exists on the safety or efficacy of use in elderly patients. Methods A retrospective chart review assessing changes in numerical symptom scores reported at clinic visits before and after medical cannabis initiation. Results There was no statistically significant difference in pain, nausea, appetite, insomnia or anxiety scores reported before and after initiation of medical cannabis. Oil was the most common form used, followed by vape, and the most common ratios used were high tetrahydrocannabinol (THC) to cannabidiol (CBD) and equal parts THC/CBD products. Conclusion This study did not find a statistically significant change in symptom scores with medical cannabis use, although further study is warranted given the limitations of the present study. Elderly patients most commonly are using equal parts THC/CBD or high THC ratio products initially.

Cannabis is becoming more popular and more available in the United States. It has been approved for use by multiple states for various conditions and several states now allow recreational cannabis. We explore the structure of cannabis distribution, the process of acquisition, outcomes, and the safety of cannabis in the United States.

Background Limited data are available on the association between clinically measured disease severity markers and quality of life (QOL) in idiopathic pulmonary fibrosis (IPF). The study examined the associations between objective disease severity metrics and QOL in a contemporary IPF population. Methods This study evaluated baseline data from patients enrolled in the multicenter, US-based Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry between June 2014 and July 2018. Disease severity metrics included FVC % predicted, diffusing capacity for carbon monoxide (Dlco) % predicted, supplemental oxygen use with activity, supplemental oxygen use at rest, and two summary scores (the Gender-Age-Lung Physiology index, based on gender, age, and % predicted values for Dlco and FVC; and the Composite Physiologic Index, based on % predicted values for Dlco, FVC, and FEV1). Multivariable adjusted regression models were used to examine cross-sectional associations between each severity measure and St. George's Respiratory Questionnaire (SGRQ) total score. Results Among 829 patients with complete SGRQ data, the median (interquartile range) SGRQ score at enrollment was 40 (26-53), with higher scores indicating worse QOL. Modest SGRQ impairments were observed with increasing Gender-Age-Lung Physiology score (2.9 [1.8-4.0] per 1-point increase] and with increasing Composite Physiologic Index scores (3.0 [2.4-3.6] per 5-point increase). Substantial SGRQ impairments were observed for oxygen use with activity (15.6 [12.9-18.2]), oxygen use at rest (16.2 [13.0-19.4]), and decreasing Dlco (5.0 [4.0-6.1] per 10% decrease in % predicted). Conclusions Objective measures of disease severity, including severity scores, physiologic parameters, and supplemental oxygen use, are associated with worse QOL in patients with IPF. Trial Registry ClinicalTrials.gov; No.: NCT01915511; URL: www.clinicaltrials.gov.

Advance care planning (ACP) discussions aim to ensure goal-concordant care for patients with serious illness, throughout treatment and especially at the end of life. But recent literature has forced the field of palliative care to wrestle with the definition and impact of ACP. Are ACP discussions worthwhile? Is there a difference between ACP discussions early in a patient’s illness versus discussions occurring later when a concrete medical care decision must be made? Here, we identify elements needed to answer these questions and describe how a multisite initiative will elucidate the value of discussing and documenting what matters most to patients.

ImportanceChanges in postsurgical opioid prescribing practices may help reduce chronic opioid use in surgical patients.ObjectiveTo investigate whether postsurgical acute pain across different surgical subspecialties can be managed effectively after hospital discharge with an opioid supply of 3 or fewer days and whether this reduction in prescribed opioids is associated with reduced new, persistent opioid use.Design, Setting, and ParticipantsIn this prospective cohort study with a case-control design, a restrictive opioid prescription protocol (ROPP) specifying an opioid supply of 3 or fewer days after discharge from surgery along with standardized patient education was implemented across all surgical services at a tertiary-care comprehensive cancer center. Participants were all patients who underwent surgery from August 1, 2018, to July 31, 2019.Main Outcomes and MeasuresMain outcomes were the rate of compliance with the ROPP in each surgical service, the mean number of prescription days and refill requests, type of opioid prescribed, and rate of conversion to chronic opioid use determined via a state-run opioid prescription program. Postsurgical complications were also measured.ResultsA total of 4068 patients (mean [SD] age, 61.0 [13.8] years; 2528 women [62.1%]) were included, with 2017 in the pre-ROPP group (August 1, 2018, to January 31, 2019) and 2051 in the post-ROPP group (February 1, 2019, to July 31, 2019). The rate of compliance with the protocol was 95%. After implementation of the ROPP, mean opioid prescription days decreased from a mean (SD) of 3.9 (4.5) days in the pre-ROPP group to 1.9 (3.6) days in the post-ROPP group (P P P = .02). There was no statistically significant difference in surgical complications. The conversion rate to chronic opioid use decreased following ROPP implementation among both opioid-naive patients with cancer (11.3% [143 of 1267] to 4.5% [118 of 2645]; P P = .02).Conclusions and RelevanceIn this cohort study, prescribing an opioid supply of 3 or fewer days to surgical patients after hospital discharge was feasible for most patients, led to a significant decrease in the number of opioids prescribed after surgery, and was associated with a significantly decreased conversion to long-term opioid use without concomitant increases in refill requests or significant compromises in surgical recovery.

Background Hospitalizations are common among patients with idiopathic pulmonary fibrosis (IPF). We investigated the impact of hospitalizations on outcomes in patients with IPF. Methods The IPF-PRO Registry is an observational US registry that enrolled patients with IPF that was diagnosed or confirmed at the enrolling center in the previous 6 months. Associations between patient characteristics and hospitalization, and between hospitalization and mortality, were analyzed using Cox regression models. Results A total of 1002 patients with IPF were enrolled into the IPF-PRO Registry. Over a median follow-up time of 23.7 months (maximum: 67.0 months), 568 patients (56.7%) had at least one hospitalization. Of these patients, 319 (56.2%) had at least one respiratory-related hospitalization and 120 (21.1%) had at least one hospitalization with ventilatory support. Younger age (HR 0.68 [95% CI 0.55, 0.84] per 5-year increase for patients Conclusions Data from the IPF-PRO Registry demonstrate that hospitalizations are common among patients with IPF. The risk of mortality during hospitalization or within 90 days of discharge was high, particularly among patients who were hospitalized for a respiratory cause or received ventilatory support. Trial registration ClinicalTrials.gov, NCT01915511. Registered 5 August 2013, https://clinicaltrials.gov/ct2/show/NCT01915511

Safe, effective, and easily implementable treatments that reduce the progression of respiratory failure in COVID-19 are urgently needed. Despite the increased adoption of prone positioning during the pandemic, the effectiveness of this technique on progression of respiratory failure among nonintubated patients is unclear.What is the effectiveness of smartphone-guided self-prone positioning recommendations and instructions compared with usual care in reducing progression of respiratory failure among nonintubated patients with COVID-19?Awake Prone Position for Early Hypoxemia in COVID-19 (APPEX-19) is a multicenter randomized clinical trial that randomized nonintubated adults with COVID-19 on 6 L/min of supplemental oxygen to receive a smartphone-guided self-prone positioning intervention or usual care. The primary outcome was the composite of respiratory deterioration (an increase in supplemental oxygen requirement) or ICU transfer. Using a Bayesian statistical approach, the posterior probability of superiority within each treatment arm (superiority threshold 95%) was calculated.The trial was stopped early for slow enrollment. A total of 293 participants were included in the modified intention-to-treat analysis (159 self-prone positioning intervention and 134 usual care). Among participants who self-reported body positioning (n = 139 [70 intervention, 69 usual care]), 71.4% in the intervention arm and 59.4% in the usual care arm attempted prone positioning. Thirty-one participants (posterior mean, 24.7%; 95% credible interval, 18.6-31.4) receiving usual care and 32 participants (posterior mean, 22.1%; 95% credible interval, 16.6-28.1) receiving the self-prone positioning intervention experienced the primary outcome; the posterior probability of superiority for the self-prone positioning intervention was 72.1%, less than the 95% threshold for superiority. Adverse events occurred in 26.9% of participants in the usual care arm and in 11.9% of participants in the intervention arm.Among nonintubated patients with COVID-19, smartphone-guided self-prone positioning recommendations and instructions did not promote strong adherence to prone positioning.ClinicalTrials.gov; No.: NCT04344587; URL: www.gov.

Palliative care has evolved to be an integral part of comprehensive cancer care with the goal of early intervention to improve quality of life and patient outcomes. The NCCN Guidelines for Palliative Care provide recommendations to help the primary oncology team promote the best quality of life possible throughout the illness trajectory for each patient with cancer. The NCCN Palliative Care Panel meets annually to evaluate and update recommendations based on panel members' clinical expertise and emerging scientific data. These NCCN Guidelines Insights summarize the panel's recent discussions and highlights updates on the importance of fostering adaptive coping strategies for patients and families, and on the role of pharmacologic and nonpharmacologic interventions to optimize symptom management.

Background: Outpatients with cancer commonly have nonmedical opioid use (NMOU) behaviors and use opioids to dull emotional and existential suffering. Buprenorphine is often used for cancer pain due to less reported euphoria when compared to other opioids. Methods: A retrospective review was done in patients who were prescribed buprenorphine for cancer pain. Pain scores were reported on a Likert pain scale of 1 to 10. Nonmedical opioid use was defined as patients taking opioids for emotional pain at or above the maximum prescribed amount. Results: For 16 patients, the mean pain score prior to buprenorphine (pain pre) was 8.3 (Standard deviation (Std) 1.6), and the mean pain score on follow-up post-buprenorphine (pain post) was 6.1 (Std 2.3) with a reduction in mean pain score (pain change) of −2.0 (Std 2.9, P = .059). Those patients without NMOU had a pain prescore of 9.5 (Std 1.0) and pain post of 4.3 (Std 2.5) with a mean pain change of −5.0 (Std 1.7, P = .20). The mean pain change in those with chemical coping (−1.3/Std 2.7), illicit drug use (−2.8/Std 1.0), or psychiatric comorbidity (−2.4/Std 2.7) were reduced after buprenorphine, however, not statistically significant. Outpatient rotation to buprenorphine was well tolerated. Conclusions: The pain score in those patients without NMOU was significantly lower after rotation to buprenorphine than those with NMOU. We deduce that in those with NMOU, it is more challenging to achieve pain relief with buprenorphine. Overall, for all patients, rotation to buprenorphine resulted in a marginally significantly reduced pain score.

Background Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease for which diagnosis and management remain challenging. Defining the circulating proteome in IPF may identify targets for biomarker development. We sought to quantify the circulating proteome in IPF, determine differential protein expression between subjects with IPF and controls, and examine relationships between protein expression and markers of disease severity. Methods This study involved 300 patients with IPF from the IPF-PRO Registry and 100 participants without known lung disease. Plasma collected at enrolment was analysed using aptamer-based proteomics (1305 proteins). Linear regression was used to determine differential protein expression between participants with IPF and controls and associations between protein expression and disease severity measures (percent predicted values for forced vital capacity [FVC] and diffusion capacity of the lung for carbon monoxide [DLco]; composite physiologic index [CPI]). Multivariable models were fit to select proteins that best distinguished IPF from controls. Results Five hundred fifty one proteins had significantly different levels between IPF and controls, of which 47 showed a |log2(fold-change)| > 0.585 (i.e. > 1.5-fold difference). Among the proteins with the greatest difference in levels in patients with IPF versus controls were the glycoproteins thrombospondin 1 and von Willebrand factor and immune-related proteins C-C motif chemokine ligand 17 and bactericidal permeability-increasing protein. Multivariable classification modelling identified nine proteins that, when considered together, distinguished IPF versus control status with high accuracy (area under receiver operating curve = 0.99). Among participants with IPF, 14 proteins were significantly associated with FVC % predicted, 23 with DLco % predicted, 14 with CPI. Four proteins (roundabout homolog-2, spondin-1, polymeric immunoglobulin receptor, intercellular adhesion molecule 5) demonstrated the expected relationship across all three disease severity measures. When considered in pathways analyses, proteins associated with the presence or severity of IPF were enriched in pathways involved in platelet and haemostatic responses, vascular or platelet derived growth factor signalling, immune activation, and extracellular matrix organisation. Conclusions Patients with IPF have a distinct circulating proteome and can be distinguished using a nine-protein profile. Several proteins strongly associate with disease severity. The proteins identified may represent biomarker candidates and implicate pathways for further investigation. Trial registration ClinicalTrials.gov (NCT01915511).

Background Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a variable clinical course and high mortality. We used data from a large national US registry of patients with IPF to investigate relationships between patient characteristics, including markers of disease severity, and mortality. Methods The analysis cohort comprised patients enrolled in the IPF-PRO Registry from its inception on 5 June 2014 to 26 October 2017. The primary criterion for inclusion in this registry is that patients must be diagnosed or confirmed with IPF at the enrolling centre within 6 months. Associations between patient characteristics and markers of disease severity at enrolment and mortality outcomes were investigated using univariable, multivariable and adjustment models. Results Among 662 patients enrolled, 111 patients died or had a lung transplant over a follow-up period of 30 months. The probability of being free of both events at month 30 was 50.6% (95% CI: 40.0, 60.2). When patient characteristics and markers of disease severity were jointly examined in a multivariable analysis, oxygen use at rest (hazard ratio [HR] 2.44 [95% CI: 1.45, 4.10]), lower forced vital capacity (FVC) % predicted (HR 1.28 [95% CI: 1.10, 1.49] per 10% decrease) and diffusion capacity for carbon monoxide (DLco) % predicted (HR 1.25 [95% CI: 1.04, 1.51] per 10% decrease) were significantly associated with increased risk of death or lung transplant. The risk of death or lung transplant increased with increasing age in patients ≥62 years old (HR 1.18 [95% CI: 0.99, 1.40] per 5-year increase), and decreased with increasing age in patients

In the era of effective antiretroviral therapy, persons living with HIV/AIDS (PLWHA) are living longer, transforming HIV from a universally fatal disease to a serious chronic illness, warranting discussions between patients and their loved ones about advance care planning (ACP). Evidence is needed on factors associated with patients' likelihood to discuss ACP with loved ones.To further characterize factors associated with successful ACP in PLWHAs with their loved ones, we examined associations between patients having ACP discussions with the need for assistance with personal care, chronic pain, life satisfaction, prior family disagreements over health care decisions, sex, age, and interference in daily routines due to memory problems.Data were from the Affirm Care study (N = 370), which examined social and environmental factors associated with health outcomes among PLWHAs and their informal caregivers.Slightly more than half of respondents discussed ACP with loved ones (57%). In adjusted analysis, higher levels of chronic pain (odds ratio [OR] = 2.09, P = 0.045), needing assistance with personal care (OR = 1.63, P = 0.023), greater life satisfaction (OR = 1.02, P = 0.002), prior family arguments over health care decisions (OR = 2.80, P 0.001), and female sex (OR = 2.22, P = 0.001) were associated with higher odds of discussing ACP with loved ones, whereas age, drug use, education level, depression, and memory problems were nonsignificant.These results suggest that interventions to increase ACP among PLWHAs and their loved ones should target males. The findings also suggest PLWHAs with chronic pain, the need for assistance with personal care, and those with a history of prior family arguments over health care decisions may be primed for ACP.

Background Performance benchmarks for the management of idiopathic pulmonary fibrosis (IPF) have not been established. We used data from the IPF-PRO Registry, an observational registry of patients with IPF managed at sites across the US, to examine associations between the characteristics of the enrolling sites and patient outcomes. Methods An online survey was used to collect information on the resources, operations, and self-assessment practices of IPF-PRO Registry sites that enrolled ≥ 10 patients. Site variability in 1-year event rates of clinically relevant outcomes, including death, death or lung transplant, and hospitalization, was assessed. Models were adjusted for differences in patient case mix by adjusting for known predictors of each outcome. We assessed whether site-level heterogeneity existed for each patient-level outcome, and if so, we investigated potential drivers of the heterogeneity. Results All 27 sites that enrolled ≥ 10 patients returned the questionnaire. Most sites were actively following > 100 patients with IPF (70.4%), had a lung transplant program (66.7%), and had a dedicated ILD nurse leader (77.8%). Substantial heterogeneity was observed in the event rates of clinically relevant outcomes across the sites. After controlling for patient case mix, there were no outcomes for which the site variance component was significantly different from 0, but the p-value for hospitalization was 0.052. Starting/completing an ILD-related quality improvement project in the previous 2 years was associated with a lower risk of hospitalization (HR 0.60 [95% CI 0.44, 0.82]; p = 0.001). Conclusions Analyses of data from patients with IPF managed at sites across the US found no site-specific characteristics or practices that were significantly associated with clinically relevant outcomes after adjusting for patient case mix. Trial registration ClinicalTrials.gov, NCT01915511. Registered 5 August 2013, https://clinicaltrials.gov/ct2/show/NCT01915511

Background Few data are available on the extent to which clinical practice is aligned with international guidelines for the management of idiopathic pulmonary fibrosis (IPF). We investigated the extent to which management guidelines for IPF have been implemented in the US IPF-PRO Registry and associations between implementation of guidelines and clinical outcomes. Methods We assessed the implementation of eight recommendations in clinical practice guidelines within the 6 months after enrollment: visit to a specialized clinic; pulmonary function testing; use of oxygen in patients with resting hypoxemia and exercise-induced hypoxemia; referral for pulmonary rehabilitation; treatment of gastro-esophageal reflux disease; initiation of anti-fibrotic therapy; referral for lung transplant evaluation. An implementation score was calculated as the number of recommendations achieved divided by the number for which the patient was eligible. Associations between implementation score and outcomes were analyzed using logistic regression and Cox proportional hazards models. Results Among 727 patients, median (Q1, Q3) implementation score was 0.6 (0.5, 0.8). Patients with an implementation score >0.6 had greater disease severity than those with a lower score. Implementation was lowest for referral for pulmonary rehabilitation (19.5%) and lung transplant evaluation (22.3%). In unadjusted models, patients with higher implementation scores had a greater risk of death, death or lung transplant, and hospitalization, but no significant associations were observed in adjusted models. Conclusions Management guidelines were more likely to be implemented in patients with IPF with greater disease severity. When adjusted for disease severity, no association was found between implementation of management guidelines and clinical outcomes.

12125 Background: While access to cannabis is prevalent among cancer patients1, unknowns persist about real-time use, perceived effectiveness in cancer pain management and impact on other medication use, including opioids. Moreover, racial disparities in cancer pain management persist2-3 with Black patients more likely to have undiagnosed pain.4 Studies on the intersection of cannabis, opioids, race and cancer pain are needed. As part of an NCI-funded initiative, we assessed the perceived effectiveness of cannabis vs. opioids for pain control by race. Methods: A survey was created by three Mid-Atlantic, cancer centers to examine self-reported cannabis use and knowledge, barriers and perceptions about cannabis among cancer patients. All sites assessed common core questions and included additional questions assessing cannabis and opioid use. Patients were eligible to participate if they were treated for cancer within the prior year. Surveys were distributed both by mail and electronically via REDCap. Results are compared separately by site. Results: In all, 2,734 patients are included in this analysis. Rates of cannabis use since diagnosis ranged from 32-41% of respondents, with similar rates of use between gender and race. Patients under age 65 more frequently reported cannabis use. Across sites, only 7-8% of patients reported currently using opioids. A sizable number patients who use cannabis reported using cannabis instead of opioids to treat pain. Of those using cannabis, most felt that cannabis was better in managing their pain than opioids, with Black patients reporting this much more frequently than White patients across two sites (Site A 62.2% vs 43.2%, Site B 77.78 vs 48.96%, Site C 50.0% vs. 65.5%). The most common reasons patients reported using cannabis instead of opioids included the perception that: “cannabis is safer” (80-82%), “cannabis is less addictive” (70-73%), and “cannabis has fewer side effects” (68-74%). Conclusions: Cannabis is frequently used by cancer patients, with many reporting use instead of opioids for pain management. Of those using cannabis for pain management, a significant majority felt that it is more effective than opioids. Black patients report cannabis as more effective than opioids for pain control more frequently than other races. Given the ongoing under-treatment of cancer pain in Black patients, more data is needed to understand whether cannabis is an effective tool to reduce disparities in cancer pain management. [Table: see text]

Context: Malignant bowel obstruction (MBO) is a complication of advanced malignancy. For inoperable patients, symptoms are often treated using analgesics, anticholinergics, and anti-emetics. There are, however, few published guidelines for the medical management of MBO. Objective: To measure the effect of the combination of dexamethasone, octreotide, and metoclopramide (“triple therapy”) in patients with MBO, compared to patients who received none of the 3 medications (“no drug therapy”). Methods: A retrospective cohort study of patients with MBO admitted in a single-center comprehensive cancer center. Patients who received dexamethasone, octreotide, and metoclopramide during their hospitalization for treatment of inoperable MBO were selected for analysis. Patients were excluded if they received a venting gastric tube. Rate of de-obstruction as well as time to de-obstruction were measured. Results: There were 20 patients identified who received all 3 drugs of interest, and 29 patients identified who received none of the 3 medications. There was no statistically significant difference in rates of de-obstruction between the 2 groups, though there was a non-significant trend toward patients who received triple therapy were more likely to reach de-obstruction, compared to patients who had no drug therapy (95% vs. 83%, p = 0.379); there was no significant difference in adjusted analysis. Conclusion: In patients with inoperable MBO, there was no statistically significant difference in rates of de-obstruction with triple drug therapy compared to patients who received none of the 3 drugs, though the study may not have been powered to detect a difference and further investigation is warranted.

Rationale Two antifibrotic medications, nintedanib and pirfenidone, have been approved for the treatment of idiopathic pulmonary fibrosis (IPF) in the US. Few data have been published on the use of these medications in clinical practice. Objective To investigate patterns of use of antifibrotic medications in the US. Methods The IPF-PRO Registry, a multicenter US registry, has enrolled patients with IPF that was diagnosed or confirmed at the enrolling center in the past 6 months. Data from patients enrolled from 5 June 2014 to 4 March 2018 were used to determine antifibrotic medication use ("treatment") in the enrollment window and a follow-up window approximately 6 months later. Associations between patient characteristics and treatment status were tested using logistic regression. Results Overall, 551 of 782 eligible patients (70.5%) were treated in the enrollment window. Younger age, lower FVC % predicted, oxygen use with activity, worse self-rated health based on the Short Form-12 (SF-12) or St George's Respiratory Questionnaire score, referral to the enrolling center by a pulmonologist, use of lung biopsy in diagnosis, and carrying a diagnosis of IPF to the enrolling center were associated with being treated. Among 534 patients treated at enrollment who had follow-up data, 94.0% remained treated in follow-up. Better self-rated health based on the SF-12 mental component score or EuroQoL score, and not using oxygen with activity at enrollment, were associated with continuing treatment in follow-up. Among 172 patients who were untreated at enrollment and had follow-up data, 29.7% started treatment in follow-up. Lower DLco % predicted, a family history of ILD, a history of sleep apnea, and a definite diagnosis of IPF at enrollment were associated with starting treatment in follow-up. Conclusions The majority of patients in the IPF-PRO Registry were receiving an approved medication for IPF at enrollment. Treatment at enrollment was associated with greater disease severity, more compromised quality of life and the use of oxygen with activity. Clinical trial registered with ClinicalTrials.gov (NCT01915511).

Rationale: Progression of idiopathic pulmonary fibrosis (IPF) is accompanied by worsening of symptoms, exercise capacity, and health-related quality of life. However, the utility of patient-reported outcomes as predictors of mortality remains uncertain. Objectives: To assess whether patient-reported outcomes are independently associated with mortality beyond clinical risk factors in patients with IPF. Methods: Data from the observational IPF Prospective Outcomes Registry were used to examine associations between patient-reported outcomes at enrollment and the composite outcome of death or lung transplant in the following year. Associations were examined using univariable models and models adjusted for age and clinical variables that have been associated with death or lung transplant in patients with IPF in this cohort (oxygen use, forced vital capacity % predicted, and diffusing capacity of the lungs for carbon monoxide % predicted at enrollment). Results: Among 662 patients, 45 died and 12 underwent lung transplant over 1 year. In the model adjusted for age and clinical variables that were associated with death or lung transplant, worse scores on the St. George’s Respiratory Questionnaire (SGRQ) total score (hazard ratio [HR], 1.22 [95% confidence interval (CI), 1.01–1.48] per 10-point increase), SGRQ activity score (HR, 1.25 [95% CI, 1.02–1.54] per 10-point increase) and SGRQ symptoms score (HR, 1.17 [95% CI, 1.01–1.36] per 10-point increase) were associated with death or lung transplant over 1 year. Conclusions: Patient-reported outcomes that assess symptoms and physical activity are independently associated with mortality in patients with IPF.

As palliative care (PC) continues its rapid growth, an emerging body of evidence is demonstrating that its approach of interdisciplinary supportive care benefits many patient populations, including in the oncology setting. As studies and data proliferate, however, questions persist about who, what, why, when, and how PC as well as the ideal time for a PC consult and length of involvement. When comparing outcomes from chemotherapy trials, it is important to consider the dosing regimens used in the various studies. In the same way, it is important to account for the “dose” of the PC interventions utilized across studies, and apples to apples comparisons are needed in order to draw accurate conclusions about PC’s benefits. Studies which include a true interdisciplinary PC intervention consistently show improvements in patient quality of life, as well as cost savings, with further study needed for other outcomes. These benefits cannot be extrapolated to care which may be labeled “palliative care,” but which does not meet the standard of true interdisciplinary PC. The ultimate question is: Does PC indeed improve outcomes?

Immune checkpoint inhibitors (ICIs) have opened an exciting chapter in the treatment of patients with advanced cancer. For the palliative care clinician, however, ICIs present several new challenges, including new ways to define treatment success, as well as treatment-related toxicities that differ in nature and timing from traditional chemotherapy. In this article, we review the mechanism of action of ICIs, as well as selected published data supporting the efficacy of ICIs in patients with advanced cancer. In addition, we summarize existing data of ICI toxicity prevalence, patterns of severity, and timing of onset. Finally, we briefly review key principles from published guidelines on the management of ICI toxicities.

Background Most states have now passed legislation mandating pulse oximetry for all newborns, or have promulgated regulations or guidelines to encourage use of routine pulse oximetry. State-based birth defects registries may be well positioned to track and evaluate critical congenital heart disease (CCHD) screening coverage and outcomes. This purpose of this study was to determine: (1) the proportion of cases detected by screening, (2) health services use by children with CCHDs during the first year of life, and (3) mortality outcomes. Methods Records of children born in 2012 to 2013 with any of seven CCHD lesions were identified in New England birth defects databases. Information was abstracted from each child's medical record. Descriptive statistics were used to report results. Results From nearly 160,000 live births, 208 CCHD diagnoses were noted in the records of 157 children. Screening was noted in 67% of records of confirmed cases of CCHDs. Data completeness varied by state; for example, information was available regarding prenatal diagnosis in 91% of records and age at first surgery in 85% among states with active surveillance compared with 35% and 75%, respectively, with passive surveillance. Documentation of screening results in medical records was inconsistent. The one year survival was 85% (77/91). Conclusion Birth defects surveillance systems can provide information on outcomes for infants with CCHDs. However, information varies by surveillance method and by hospital practices. Engaging hospitals in standardizing recording procedures and enhancing training and quality control could increase the value of birth defects registries records in assessing outcomes for children identified through CCHD screening. Birth Defects Research 109:1414–1422, 2017.© 2017 Wiley Periodicals, Inc.

12082 Background: Treating patients with metastatic cancer (MCA) requires timely integration of palliative and hospice care (PHC). The Surprise Question (SQ-“Would you be surprised if your patient died in the next year?”) may help predict short-term mortality in patients with chronic disease and prompt initiation of PHC. There limited data on the utility of the SQ in oncology and none controlling for age and cancer site (CS). This study evaluates the SQ in MCA. Methods: SQ data were collected using a clinical oncology pathway program (COP) in which the treating oncologist is required to answer the SQ for all patients with MCA. The cohort includes MCA patients with SQ entries from 5-1-2018 to 4-30-2019 providing cohorts with 6- and 12-months follow-up (f/u) from the date of the SQ. Vital status was determined as of 1-15-20. Over 90% of deaths are reported with 3 months of death. Models using SQ response (yes/no), cancer site and age were tested with logistic regression (LR) with Akaike Information Criterion (AIC) for model selection (lower number best model) and ANOVA to assess the value of the SQ in predicting mortality. Results: There were 655 and 1276 patients with MCA in COP with 1 year and 6 months f/u from the SQ, respectively. The proportion with the SQ response “No” (shorter expected survival) varied by cancer site (range 19% – 82% - e.g. breast 42%; pancreas 81%). The SQ was the best predictor compared to cancer site and age for 6 and 12-month mortality (LR Model A – lowest AIC). The odds ratio of death at one year from SQ-No vs. SQ-Yes was 3.8 (95% CI 2.7, 5.4) with a strong association between SQ response and 1-year and 6-month mortality (p < 0.001). See Table. Conclusions: Oncologist assessment of survival expectancy by the SQ in MCA is a strong predictor of mortality beyond cancer site and age. The treating oncologist’s response to SQ can serve as a simple and reliable predictive tool to identify those with MCA likely to die sooner who may benefit from timely referral to PHC. [Table: see text]

ObjectiveTo evaluate the relationships between maternal fish consumption and pregnancy outcomes in a large, population-based sample of women in the USA.DesignWe collected average fish consumption prior to pregnancy using a modified version of the semi-quantitative Willett FFQ. We estimated adjusted OR (aOR) and 95 % CI for associations between different levels of fish consumption and preterm birth (SettingThe National Birth Defects Prevention Study (NBDPS).SubjectsControl mother–infant pairs with estimated delivery dates between 1997 and 2011 (n 10 919).ResultsNo significant associations were observed between fish consumption and preterm birth or early preterm birth (aOR = 0·7–1·0 and 0·7–0·9, respectively). The odds of having an SGA infant were elevated (aOR = 2·1; 95 % CI 1·2, 3·4) among women with daily fish consumption compared with women consuming fish less than once per month. No associations were observed between other levels of fish consumption and SGA (aOR = 0·8–1·0).ConclusionsHigh intake of fish was associated with twofold higher odds of having an SGA infant, while moderate fish consumption prior to pregnancy was not associated with preterm or SGA. Our study, like many other studies in this area, lacked information regarding preparation methods and the specific types of fish consumed. Future studies should incorporate information on nutrient and contaminant contents, preparation methods and biomarkers to assess these relationships.

Children with disabilities are at an increased risk for maltreatment. However, little is known about the risk of maltreatment in children with specific types of birth defects. This study was conducted to determine whether the risk and predictors of maltreatment in children 2 to 10 years of age differ between those without and with specific birth defects: Down syndrome, cleft lip with/without cleft palate, and spina bifida. State administrative and United States Census data were linked to identify study groups, variables of interest, and outcome measures. Kaplan-Meier and multivariate Cox proportional hazard analyses were used to identify study groups and variables associated with an increased risk for maltreatment. The prevalence of substantiated maltreatment was consistently highest among children with cleft lip with/without cleft palate. After adjusting for birth-level factors, children with Down syndrome and cleft lip with/without cleft palate were 34% and 26% more likely to have been maltreated than those without birth defects, respectively. In all three birth defect groups, the risk of medical neglect was higher (relative risks ranged from 3 to 11) than in the unaffected group. The factors associated with increased risk for maltreatment were similar across all groups. Of note, parity, maternal education, and maternal Medicaid use at birth were all associated with greater than 2-fold increased risk for maltreatment. Our findings suggest that the families of children with birth defects may need support services throughout early childhood to help families cope with the needs of their children and reduce the risk of maltreatment.

Background State-specific information about hospitalizations of children with birth defects can improve understanding of changes in occurrence, treatment practices, and health care financing policies. This study analyzed aggregated data on hospital charges and length of stay for a large, diverse population. Methods We extracted hospitalization data for children diagnosed with birth defects from the Texas Hospital Inpatient Discharge Public Use Data File (2001-2010). Analyses compared total charges and length of stay for children with and without a diagnosis code of any birth defect among 45 standard categories. We also examined trends for total charges by expected payer type. Results In Texas, 431,296 hospital stays were reported for children with birth defects, with total charges of $24.8 billion. Mean hospital stay for children with birth defects was more than twice that of those without, whereas mean of hospital total charges was approximately six times greater. Pyloric stenosis accounted for the largest number of hospitalizations, followed by certain cardiac defects. Pediatric hospitalizations for birth defects increased 273.7%, compared with a 214.7% increase overall. The percentage of charges with Medicaid as expected payer (2004-2010) ranged from 56.5 to 62.0%. Conclusion Charges associated with these conditions are far greater than those associated with pediatric hospitalizations for other causes, whether in the newborn period or beyond. However, these charges vary depending on specific diagnoses, expected payer source, and year of treatment.

BACKGROUND AND OBJECTIVES: Children with disabilities are at an increased risk for maltreatment. However, the risk of maltreatment is unknown for children with specific types of birth defects. This study was conducted to determine whether the risk and predictors of maltreatment differ between children with and without 3 birth defects: Down syndrome, cleft lip with/without cleft palate, and spina bifida. METHODS: This population-based study of substantiated childhood maltreatment was conducted in Texas from 2002 to 2011. Linked data were used to describe the risk and types of maltreatment that occurred before age 2 years in children with and without specific birth defects. Poisson regression was used to identify predictors of maltreatment and assess differences in those predictors between children with and without these specific birth defects. RESULTS: The risk of maltreatment (any type) in children with cleft lip with/without cleft palate and spina bifida was increased by 40% and 58%, respectively, compared with children with no birth defects. The risk of any maltreatment was similar between children with Down syndrome and unaffected children. Across birth defect groups, the risk of medical neglect was 3 to 6 times higher than in the unaffected group. Child-, family-, and neighborhood-level factors predicted maltreatment in children with and without birth defects. CONCLUSIONS: The overall risk of substantiated maltreatment was significantly higher for some but not all birth defect groups. The factors associated with increased risk were similar across groups. Enhancement of existing maltreatment prevention and early intervention programs may be effective mechanisms to provide at-risk families additional support.

Study Objectives We sought to determine whether selected periconceptional health behaviors that influence risk for birth defects differ between older and younger adolescents and whether pregnancy intention predicts more positive preconception health behaviors among teens. Design and Participants We analyzed interview responses from 954 adolescent control group participants from the National Birth Defects Prevention Study who delivered live infants during 1997-2007. Main Outcome Measures Adjusted odds ratios (aORs) and 95% confidence intervals (CIs) were calculated for factors of interest by age categories (13-15, 16-17, and 18 years, relative to 19 years). To construct a composite periconceptional behavior index, we summed the following healthy behaviors: nonsmoker, nondrinker, folic acid supplementation, and eating 5 or more servings of fruits and vegetables per day. Results Analyses indicated that women in the youngest group (13-15 years of age) were more likely to be Hispanic (aOR 2.83, 95% CI 1.40-5.70) and less likely to engage in some unhealthy pregnancy-related behaviors compared with 19-year-olds, such as smoking (aOR 0.45, 95% CI 0.20-0.99) and being overweight or obese (aOR 0.32, 95% CI 0.16-0.61). However, they were also less likely to have taken periconceptional folic acid (aOR 0.44, 95% CI 0.21-0.90). About one-third of teen mothers indicated that their pregnancies had been intended. Among 18- and 19-year-olds, this predicted a higher mean value for the composite periconceptional behavior index (2.30 versus 1.94, P ≤ .01). Conclusions Teen mothers are not a homogeneous group. Each age subgroup presents varied demographic and behavioral factors that put them at varying levels of risk for birth defects. Furthermore, caregivers should not assume that teens do not plan pregnancies or that they need not be informed of the importance of periconceptional health.

Context Despite improvements in end-of-life care, some unrelieved suffering persists for patients with advanced illness and their family members. Hospice and palliative care services can reduce suffering, but these services remain under-used. Objectives To investigate how patients with advanced illness and their primary caregivers experienced and responded to health care needs and decision making and how some dyads moved toward comfort-focused care. Methods This was a qualitative study using the grounded theory method for sample selection, data collection, and analysis. Dyadic semi-structured interviews were audio-recorded and transcribed for analysis. Twenty-two participants, 12 patients and 10 family surrogates, provided 16 interviews for this study. Results Participants engaged in a process of contending with advanced illness. The major phases comprising this process were suffering, struggling, and settling. Struggling included enduring the experience and fighting the illness. During the phase of settling, the focus shifted away from curative efforts and toward supportive care. Conditions that facilitated the movement into this phase included receiving clear and consistent information about the patient's health status, trusting health care providers, having attended to advance care planning in some form, and being aware of and able to acknowledge the terminal nature of the illness. Conclusion Findings from this pilot study offer a preliminary theoretical model to enhance the understanding of patient and family caregiver needs during advanced illness. Awareness of their perspective can inform the timing and content of clinicians' communication and interventions.

Background: Despite public health campaigns encouraging women to take a daily folic acid supplement, the proportion of reproductive age women, in the United States, who comply with this recommendation is less than optimal. The objective of this analysis was to identify predictors of preconceptional folic acid-containing supplement use to define subgroups of women who may benefit from targeted folic acid campaigns. Methods: This study included 6570 mothers of live born infants from the control population of National Birth Defects Prevention Study (1997–2005). Logistic regression analyses were used to identify predictors of preconceptional folic acid supplementation. A classification and regression tree (CART) analysis was used to define subgroups of women with different patterns of preconceptional folic acid supplementation. Results: Race/ethnicity, education, age at delivery, nativity, employment, income, number of dependents, smoking, and birth control use were significantly associated with preconceptional folic acid-containing supplement use. Based on a CART analysis, education, race/ethnicity, and age were the most distinguishing factors between women with different preconceptional supplementation patterns. Non-white women with

In order to translate research findings into effective prevention strategies, it is important to understand people's beliefs about the causes of poor health outcomes. However, with the exception of knowledge and beliefs about folic acid supplementation, little is known regarding women's causal attributions women regarding birth defects. We employed Attribution Theory constructs to analyze open-text interview responses from 2,672 control mothers in the National Birth Defects Prevention Study who gave birth in 1997-2005. Common themes included use of alcohol, tobacco, illicit drugs, and medications during pregnancy. Stress and emotional upset were also suggested as possible causes of birth defects. Genetic- and heredity-related responses were more likely to be mentioned by Asian/Pacific Islander women compared to non-Hispanic Whites. Hispanic women were less likely to suggest several specific possible teratogens, such as paint, pesticides, or other chemicals, but were more likely to suggest events occurring during childbirth. Differences also emerged among ethnic groups for theoretical constructs, although most responses were categorized as controllable, changeable over time, and with an internal locus of causality.